POS1306 EXTREME PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND CERVICAL ADENITIS (PFAPA) ARE A SUBSET OF PFAPA PATIENTS WITH FLARES THAT OCCUR MORE THAN TWICE A MONTH.
Authors: M. Broide, Y. Levinsky, G. Amarilyo, R. Tal, L. Harel, Y. Butbul, S. Abu Ahmad, S. Shoham
Journal: Annals of the Rheumatic Diseases (2022)

Background The febrile episodes of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome occur, by definition, every 2 to 8 weeks. However, a subset of our patients appears to experience attacks at an even higher rate of more than twice a month, and was therefore named extreme PFAPA, or ePFAPA group. Objectives To characterize this group both demographically and clinically in order to compare them to the non-extreme PFAPA (nPFAPA) group. Methods We retrospectively reviewed the medical records of patients with PFAPA that were treated in the Schneider Children’s Medical Center of Israel from 3/2014 to 4/2021. Patients with concomitant familial Mediterranean fever were excluded. Thereafter the ePFAPA and nPFAPA groups were compared using Wilcoxon rank sum, Pearson’s chi-squared and Fisher’s exact tests. Results 47 patients (12.9%) out a total of 365 PFAPA patients were included in the ePFAPA group. Compared to the nPFAPA group, ePFAPA patients had earlier disease onset (age 1.7 vs 2.96 years, P&lt;0.001) and diagnosis (age 2.94 vs 5.02 years, P&lt;0.001). Moreover, after initiation of an abortive treatment with corticosteroids, ePFAPA patients had higher increased flares frequency (72% vs 40%, P&lt;0.001) and were treated with colchicine prophylaxis more often (67% vs 26%, P&lt;0.001). Other clinical and demographic aspects were not significantly different between the two groups. Conclusion EPFAPA patients are a subset of patients who have earlier onset and diagnosis of PFAPA, and also increased flares frequently after abortive therapy with steroids. Current study is underway to describe the long-term outcome of this group. References [1]Periodic Fever, Aphthosis, Pharyngitis, and Adenitis Syndrome: Analysis of Patients From Two Geographic Areas Batu E, Kara Eroğlu F, Tsoukas P, Hausmann J, Bilginer Y, Kenna M, Licameli G, Fuhlbrigge R, Özen S, Dedeoğlu F Arthritis Care and Research (2016) 68(12) [2]POS1326 FAMILIAL PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND ADENITIS (PFAPA) SYNDROME; IS IT A SEPARATE DISEASE? Butbul Y Annals of the Rheumatic Diseases (2021) 80(Suppl 1) [3]PFAPA syndrome is not a sporadic disease Cochard M, Clet J, Le L, Pillet P, Onrubia X, Guéron T, Faouzi M, Hofer M Rheumatology (2010) 49(10) [4]PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment Peridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I, Houlakis M, Parpounas K International Journal of Pediatric Otorhinolaryngology [5]The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome Harel L, Hashkes P, Lapidus S, Edwards K, Padeh S, Gattorno M, Marshall G Journal of Pediatrics (2018) 193 [6]Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome Wurster V, Carlucci J, Feder H, Edwards K Journal of Pediatrics (2011) 159(6) [7]Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcome Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, Migdal A, Pras M, Passwell J Journal of Pediatrics (1999) 135(1) [8]Risk factors for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: a case-control study Kettunen S, Lantto U, Koivunen P, Tapiainen T, Uhari M, Renko M European Journal of Pediatrics (2018) 177(8) [9]A clinical review of 105 patients with PFAPA (a periodic fever syndrome) Feder H, Salazar J Acta Paediatrica, International Journal of Paediatrics Disclosure of Interests None declared